A Mother’s Argument for Patient Self-Advocacy in Rett Syndrome Using an Eye-Tracking Communication Device

I jerked awake at 1 AM to the sound of frenzied footsteps and insistent beeping in my five-year-old daughter Kalika’s hospital room. She lay in deep slumber, recovering from gastrostomy surgery. Two nurses hovered at the foot of her bed with deep frowns and voices betraying concern. Kalika’s blood pressure had plummeted alarmingly, and she appeared unresponsive.

After several minutes of prodding, she opened her eyes. The head nurse turned to me and said, “We need to see if she’s alert. Can you get her to respond on her device?”

I nodded and grabbed Kalika’s eye-tracking communication device. Holding the device over her, I asked her how much pain she felt. Kalika scanned the “Pain” page in her speech software and said: “Some pain.”

The nurse turned to me and said: “Can you ask her another question? Ask her how she feels.” I flipped to the “Feelings” page and asked, “How do you feel?” Kalika surveyed the screen and said: “Tired, tired.”

Satisfied that Kalika was in fact responding appropriately and consistently, the nurse said, “We’re good. Great job!”

Incorporating patient perspectives can be transformative.

Patient input is critical in managing care in postsurgical situations. It was extraordinary that Kalika was able to offer some that night. She has Rett syndrome, a rare neurological disorder that renders her unable to speak or to use her hands in a functional manner. Both these symptoms lead to profound communication challenges.

Historically, individuals with Rett syndrome have been denied input in their own care. Cutting-edge eye-tracking augmentative and alternative communication (AAC) technology now offers the opportunity to tap into patient perspectives in ways that were unimaginable until recently.

This technology is propelled by intentional eye gaze: the devices allow users to control a computer with eye movements to generate speech. Kalika’s eyes function like a mouse. Installed within the computer is software that she uses to talk. Whenever she wants to say something, she knows she has to look at the word or phrase for 0.4 seconds (a time we set), and then the computer speaks that word for her. Such devices have transformative potential in medical settings and are covered by insurance (in many instances) as medical necessity.

Little research to date.

However, the use of such devices is studiously ignored in related medical scholarship. No previous study, in fact, has explored how these can be used for providing medical information in Rett syndrome. While only a minority of patients have access to this technology, it is a growing population within the community.

Part of the problem, regrettably, is the pervasive scholarly suspicion that individuals with Rett syndrome are cognitively immature and incapable of offering valuable input in their care. As a scholar-mom, I have been conducting research in this area with a team of investigators. We have compelling evidence that Kalika is highly sophisticated in her navigation of medical encounters using her device. That moment in the hospital is only one of many such moments.

A few hours prior to that moment in that hospital room, Kalika had been distressed, crying in pain. During a nurse check-in, she complained of being “uncomfortable” and feeling “lots of pain.” On the strength of those complaints, she was administered morphine. Interestingly, it was the only day around her surgery that Kalika chose the phrase “lots of pain”; over the next few days, she only referred to “some pain” or “no pain” when asked.

It so happened that we had a repeat of that first blood pressure crash moment a few hours afterward, when the nurse again asked me to question her with the device to assess her alertness. That time, Kalika stated that she felt “lots of pain” and was “sleepy.” The nurse later told me that if Kalika had not responded appropriately in those terrifying moments, they would have called the code team. The stakes were that high.

The untapped potential of this communication technology.

As hard as it was to hear of Kalika’s expression of pain through her device, it was gratifying to me that she had a medium for its expression. There was precedent for her use of this device to share important information about her condition. When she had daily seizures for months, she would alert us regularly that they were about to occur, asking for our help in stopping them. She also impressed her ophthalmologist during eye exams by matching letters on the eye chart using the “Alphabet” page on her device.

These are only some noteworthy instances; as mother, I experience the marvel of her self-expression every single day, in every area of life. By the time we left the hospital a few days later, the legend of the little girl who spoke with her eyes rang throughout her floor. The fact that those nurses, and indeed my daughter’s extended medical and therapeutic team, listened to her has been heartwarming. They have trusted that a little girl with a brain disorder knows her own body best. That she could best communicate about it. And they are right.

It is my hope that the entire Rett syndrome medical community, relevant drug development teams, and scholars catch up with the magic of communication in Rett syndrome. The potential of patient input is already here: it is merely a question of opening one’s eyes to it.

Usree Bhattacharya is an associate professor of language and literacy education at the University of Georgia, Athens.